Pilomatrix carcinoma

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Overview

Pilomatrix carcinoma (also called pilomatrical carcinoma or malignant pilomatrixoma) is a very rare type of skin cancer that starts in the hair follicle. Specifically, it grows from cells that normally form the root sheath of hair. This cancer most often appears as a hard lump or mass under the skin, usually on the head, neck, or upper body. It can look similar to a benign (non-cancerous) growth called a pilomatrixoma, which makes it tricky to diagnose without a tissue sample. Pilomatrix carcinoma tends to grow slowly but can be locally aggressive, meaning it can invade nearby tissues. In some cases, it can spread to lymph nodes or other parts of the body, though this is uncommon. The tumor is usually firm, may feel calcified (hard like bone), and can sometimes ulcerate or break through the skin surface. Treatment is primarily surgical. The main goal is to remove the tumor completely with clear margins, meaning no cancer cells are left at the edges. Because this cancer is so rare, there are no standard chemotherapy or radiation protocols specifically approved for it, though these may be used in advanced cases. Early detection and complete surgical removal offer the best outcomes. Regular follow-up is important because the cancer can come back locally after surgery.

Also known as:

Key symptoms:

Hard lump or mass under the skinLump on the head, neck, face, or upper bodySkin that feels calcified or stony over the lumpSlow-growing bump that may suddenly grow fasterSkin ulceration or open sore over the lumpRedness or discoloration of the skin over the tumorLump that feels attached to deeper tissueSwollen lymph nodes near the tumor in advanced casesRecurrence of a lump after previous removal of a similar growth

Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Pilomatrix carcinoma.

View clinical trials →

No actively recruiting trials found for Pilomatrix carcinoma at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Pilomatrix carcinoma community →

No specialists are currently listed for Pilomatrix carcinoma.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Pilomatrix carcinoma.

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Community

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Latest news about Pilomatrix carcinoma

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.Was the tumor completely removed with clear margins, and what does that mean for my risk of it coming back?,How often do I need follow-up appointments, and what will you check for at each visit?,Should I have imaging tests like CT or MRI to check if the cancer has spread?,Is radiation therapy recommended in my case, and what are the benefits and risks?,Are there any genetic factors I should know about that could affect my family members?,What signs or symptoms should prompt me to contact you immediately between appointments?,Are there any clinical trials or research studies I might be eligible for?

Common questions about Pilomatrix carcinoma

What is Pilomatrix carcinoma?

Pilomatrix carcinoma (also called pilomatrical carcinoma or malignant pilomatrixoma) is a very rare type of skin cancer that starts in the hair follicle. Specifically, it grows from cells that normally form the root sheath of hair. This cancer most often appears as a hard lump or mass under the skin, usually on the head, neck, or upper body. It can look similar to a benign (non-cancerous) growth called a pilomatrixoma, which makes it tricky to diagnose without a tissue sample. Pilomatrix carcinoma tends to grow slowly but can be locally aggressive, meaning it can invade nearby tissues. In som

How is Pilomatrix carcinoma inherited?

Pilomatrix carcinoma follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Pilomatrix carcinoma typically begin?

Typical onset of Pilomatrix carcinoma is adult. Age of onset can vary across affected individuals.